2024 Prof. Dr.

Balkees Taha

Odontogenic tumors D16.5 mand, D16.4 max: C41.1, C41.0

Odontogenic tumors derive from tooth forming structure:

1.Remnants of the dental lamina (rests of Serres),
2.Reduced enamel epithelium,
3.Remnants of Hertwig's root sheath (rests of Malassez),
4.Tooth germ (Enamel organ, Dental papilla, Dental sac).
Odontogenic tumors occur only in jawbones.

Classification of odontogenic tumors (WHO 2017)

A-Benign odontogenic tumors:
1- Epithelial lesions:
a. Without odontogenic mesenchyme (Ameloblastoma, Calcifying epithelial odontogenic
tumor, Adenomatoid odontogenic tumor, Squamous odontogenic tumor)
b. With odontogenic mesenchyme (mixed) (Ameloblastic fibroma, Primordial odontogenic
tumor, Dentinogenic ghost cell tumor, Odontoma)
2- Mesenchymal lesions: (Odontogenic fibroma, Odontogenic myxoma/myxofibroma, Cementoblastoma,
Cemento-ossifying fibroma)

B-Malignant odontogenic tumors:

a. Odontogenic carcinoma: (Ameloblastic carcinoma, Primary intra-osseous squamous cell
carcinoma, Clear cell odontogenic carcinoma, Ghost cell odontogenic carcinoma,
Sclerosing odontogenic carcinoma)
b. Odontogenic carcinosarcoma.
c. Odontogenic sarcoma: (Ameloblastic fibrosarcoma, Ameloblastic fibro-odontosarcoma)

Ameloblastoma: 9310/0
It is a benign neoplasm of odontogenic epithelium, (origin) derived from enamel organ, odontogenic rests (rests of
Malassez, rest of Serres), reduced enamel epithelium and epithelial lining of the odontogenic cyst.
Pathogenesis: The mechanism for growth and invasion include; bone resorbing factors, anti-apoptotic proteins, and
interface proteins. Ameloblastomas, however, have a low proliferation rate. Mutations of the P53 gene do not appear
to play a role in the development or growth of ameloblastoma.
Behavior: It is slowly growing, locally aggressive growth produces bone expansion and facial deformity. It has a high
recurrence rate, no metastasis, it presents in 3 biological subtypes:
1. Solid/ multicystic/ polycystic/ common ameloblastoma
2. Unicystic type: is a variant of intraosseous ameloblastoma that occurs as a single cystic cavity, with
or without luminal proliferation
3. Extraosseous/peripheral type: is a benign tumor that occurs in the soft tissues of the gingiva or
edentulous alveolar area.
4. Metastasizing ameloblastoma: is an ameloblastoma that metastasizes (lung, lymph node) despite its
benign histological appearance.
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Common (polycystic) ameloblastoma:

It is the most common type; occurs in a patient over 25 years (20-40y). It most commonly locates in the
mandible, with 75% occurring in the molar and ascending ramus areas. No
significant sex or race predilection exists. Characterized by cortical bone
expansion, having a thin outer shell of bone that is cracks easily when palpated
and this diagnostic sign referred to as (egg-shell cracking).
Radiographic:
Multilocular radiolucency described as soap bubbles. Poorly-defined
margin and size of the lesion is difficult to determine (it does not exhibit a
distinct line of demarcation). Root resorption is uncommon.
Differential Diagnosis:, odontogenic myxoma, dentigerous cyst, OKC, GCG,
haemangioma, histiocytosis.

Histopathology:
The characteristic feature is the ameloblast-like cells which are the palisade columnar basal cell with reverse
polarization (nucleus away from basement membrane). It may arrange in the following form:
1. Follicular pattern: it resembles the early stage of tooth development. It consists of odontogenic epithelium in the
form of an island, strand arranged in fibrous connective tissue stroma. These islands
consist of the outer border of palisade ameloblast-like cells and central cells loosely
arranged and widely separated triangular shaped cells that are similar to (stellate
reticulum-like). The central area may undergo degeneration and form microcyst.

2. Acanthomatous pattern: central cells in the island transformed to squamous cells and
produce keratin.

3. Granular cell variant: the central cells are swollen and densely packed with
eosinophilic granules.
Types 2 and 3 occur within follicular pattern formation.

4. Plexiform pattern: it consists of odontogenic epithelium that arranged in a fishnet or mesh pattern strands. Large and
small cyst-like areas are present. They result from degeneration of the epithelium and connective tissue stroma.

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5. Basal cell variant: its epithelial cells are densely-packed with cuboidal basaloid cells exist in thin strand without
stellate reticulum.

6. Desmoplastic ameloblastoma: contain small islands or strands of darkly stained cuboidal cells in the dense fibrous
stroma. It tends to penetrate the surrounding bone, so it is difficult to detect a clear margin of the lesion, so it is the
most difficult to treat.

Treatment: it never depends on the histological pattern, all types are locally invasive, so extensive surgical removal
(block resection) recommended. The lesion is radioresistant.

1. Unicystic ameloblastoma:
It is 10-15% of all ameloblastoma intraosseous lesions. It occurs in young patient 16-20y as asymptomatic
painless swelling of the jaw, commonly associated with dentigerous cyst with severely displaced impacted third molar
90% in the mandibular posterior region, lesions commonly occur in the mandible than in the maxilla.

Radiographic: typically, it appears as unilocular

radiolucency, has well-demarcated borders with
tooth crown (unerupted third molar).

Histopathology:
The lesion consists of a dense, uniformly thickened, fibrous connective tissue capsule, surrounding a fluid-
filled lumen. The epithelial lining of the lumen is uniform in thickness and has a hyperchromatic layer of palisaded
basal cells exhibit reversed polarization of the nucleus; the remaining cells are stellate reticulum-like.
The epithelial lining may produce papillary projection extended in to the lumen and referred as intraluminal
unicystic ameloblastoma. If the projection extends to the capsular tissue it termed a mural unicystic ameloblastoma.

Treatment: for intraluminal, enucleation is sufficient. If it is mural (extends into the wall) interface with the marginal
bone resection is needed and follow up to ensure adequate removal.

3. Peripheral ameloblastoma (extraosseous):

Uncommon (1%) it is painless, firm sessile nodules of the gingival that has a smooth
surface and normal coloration. It is small in size but continuously growing.
Differential Diagnosis: Odontogenic fibroma.
Radiographic: occasionally superficial saucerization of the cortical plate. Tooth separation may occur if the lesion
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located in the interdental papilla areas.

Histopathology: Resemble follicular pattern (acanthomatous variant) lesion surround by fibrous tissue.

Treatment: local excision with a small margin of healthy tissue.

4. Malignant ameloblastoma
it is metastasizing ameloblastoma included as a type of benign conventional ameloblastoma
Clinical: it observed in patients with age range 4-75 years (mean age, 30 years). Nearly one-third of patients with
metastases, become apparent 10 yrs after treatment of the primary tumor. Metastases are most often seen in the lungs
then secondly to the cervical lymph nodes. Spread to vertebrae and other bones are occasionally.

Radiograph: similar to typical non-metastasizing ameloblastomas.

Histopathology: like ameloblastomas with a completely benign local course.
Prognosis: has a poor prognosis. 50% of patients died of their disease.

Calcifying epithelial odontogenic tumor (Pindborg tumor):

It is rare (1%) benign epithelial odontogenic tumor. It secretes an amyloid protein that tends to calcify. It is locally
aggressive, not capsulated, arise (origin) from epithelial rests of the dental lamina or the reduced enamel epithelium, it
may be of 2 types: central (intraosseous) or peripheral (extraosseous).
The age ranged 30-50y, there is no sex predilection, appear as painless slow growing swelling,
67-75% occurs mandibular posterior areas (molar), while the peripheral type seen mostly at the anterior area of the
mouth as nonspecific gingival mass.

Radiographic: Diffuse (ill-defined border) unilocular or multilocular radiolucency with flecks of calcification
structures of varying size and associated with an unerupted displaced molar.
Peripheral type sometimes lesions exhibit superficial cortical erosion.

Differential Diagnosis: CEOC, adenomatoid odontogenic tumor, ossifying fibroma, osteoblastoma.

Histopathology:
It consists of sheets of polyhedral cells with prominent intercellular bridges, nuclear pleomorphism,

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prominent nucleoli, and hyperchromatism, rare mitosis. There is a pool of homogenous eosinophilic material and
spherical concentric rings (Liesegang rings) of calcification scattered through the epithelium and connective tissue
(amyloid-like material).

The peripheral types have fewer tendencies for calcification but have prominent clear cell cytoplasm variant.
Treatment: It is less aggressive than ameloblastoma, so local resection including normal soft tissue and bone
recommended. Recurrence rate is 15%, prognosis good.

Adenomatoid odontogenic tumor:

Uncommon, it considered as hamartoma. It arises from reduced enamel
epithelium, its benign non-aggressive growth. Seen in young patient 10-19 yrs,
females are more affected, and it mostly occurs in the anterior area of the jaw
with an impacted tooth (canine) mainly maxilla.
Radiographic: Well circumscribed unilocular radiolucency involves the crown
of an impacted tooth extends beyond the CEJ and may contain fine calcification
(differed from the dentigerous cyst).
Histopathology: The mass consists of thick fibrous capsule surrounding a nodular pattern of epithelial cells (the
nodule composed of spindled epithelial cells), and scattered ductal structure lined by columnar cells, and spherical
calcification and occasionally hyaline material scattered in a fibrous stroma.
Treatment: Easley enucleated because it is capsulated, no recurrence.

Squamous odontogenic tumor:

It is rare. It arises from the remnant of dental lamina, rests of Malassez, overlying epithelial associated
with lateral root surface of the erupted tooth. It is slow growing. Occur at the anterior region to molars, equally
distributed between the maxilla and mandible, at any age mainly third decade as painless gingival swelling or as local
tenderness and mobility of teeth.

Radiographic: Unilocular radiolucency lateral to roots result in teeth

separation but no root resorption. It is well demarcated.
Differential Diagnosis: Lat. Od cyst, vertical PD bone loss.

Histopathology: Round and elongated islands of stratified squamous epithelium in cellular fibrous connective tissue
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stroma (no polarization in epithelium), many of the islands have central areas of microcyst formation

Treatment: Local excision or curettage. No recurrence.

Note. Always maxillary odontogenic tumors with local aggressiveness are more severe than mandible because of the
porous, spongy nature of maxilla and presence of adjacent vital organs and structures.

Odontogenic fibroma: 9321/0

Mostly occur in females, 60%in maxilla in anterior to molars, half of them posterior to molar associated with
localized bone expansion or loosening of teeth, occur in extraosseous (peripheral) and intraosseous.
1. Peripheral odontogenic fibroma (extraosseous):
Focal gingival growth similar to peripheral fibroma. It may be of normal coloration or erythematous when
ulceration occurs.
Radiographic: because lesion located in soft tissue does not appear in the radiograph.

Histopathology: the lesion is composed of a mixture of dense connective tissue that separates
localized zones of myxomatous or loose connective tissue that is adjacent to the small epithelial
island found in it.

Treatment: local excision to superficially remove the lesion not extending the deep margin.
2. Central odontogenic fibroma (intraosseous) (WHO type):
Lesions are usually asymptomatic, painless swelling commonly located in the mandible

Radiographic: small lesions are well defined, unilocular radiolucency often associated with the apical area of erupted
teeth. Large lesions are multilocular; root resorption is common.

Histopathology: Cellular fibrous connective tissue with collagen fibers (interlacing

bundles), thin strands of odontogenic epithelial scattered widely. The fibrous tissue may be
myxoid to hyalinized, calcified material is also present.
Differential Diagnosis: CEOT, ameloblastoma, cementifying fibroma.
Treatment: enucleation and curettage, prognosis good.

Odontogenic myxoma: 9320/0

It is un common intraosseous lesion and locally aggressive behavior, equally distributed between the mandible
and maxilla occur at any area of the jaw, not capsulated, tends to infiltrate surrounding bone.
Young patient 25-30ys there is no sex predilection. Small lesions are asymptomatic but larger lesion associated with
painless expansion.

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Radiographic: unilocular or multilocular radiolucent defect with irregular or scalloped

margins, thin trabeculae may be arranged at right angle one to another seen within
radiolucency. Large myxoma of the mandible may show soap-bubble pattern resemble
ameloblastoma.

Histopathology: grossly it is gelatinous mass, under a microscope, it

consists of loosely arranged stellate, spindle-shaped and round cells in
an abundant, loose myxoid stroma that contain few collagen fibrils
(acid mucopolysaccharide mainly hyaluronic acid and chondroitin
sulfate). Island of odontogenic epithelium may be seen in the myxoid
ground substance.

Treatment: small unilocular lesion treated by local curettage followed by chemical cautery of the bony walls, but
most lesions require block resection. Recurrence occurs due to the gelatinous nature of the lesion. It is important to
remove an intact specimen to reduce the chances of recurrence. Also, the myxomatous tissue penetrates the trabecular
spaces so this cause difficulty in conservatively removing the lesion. Prognosis? Try to answer.

Cementoblastoma: 9273/0
A benign neoplasm of cementoblast. It is a rare lesion that occurs in the 2nd and third decades with a peak
incidence around 19 years. Nearly all tumors occur in the molar and premolar area with lesion attached to the apical
third of one of the roots. Patient has pain being more intense with palpation, and the teeth
remain vital.
Radiographic: unilocular and well demarcated, it is either
1) completely radiolucent,
2) mixture of radiolucent and radiopaque, or
3) entirely radiopaque.
Radiopaque lesion exhibits a peripheral zone of radiolucency continuous
with the normal periodontal ligament space of the unaffected areas of the
tooth. Roots adjacent to the expanding lesion often exhibit resorption of
their apical third.
Histopathology:
Calcified cementum-like tissue is deposited in thick trabeculae on an intact
or partially resorbed root. The formed cementum is strongly basophilic
and shows numerous irregular reversal lines resembling Paget's disease of bone.
The histologic features are similar if not identical to those of an osteoblastoma but with attachment to a tooth root.

Treatment: enucleation with the removal of the associated tooth.

Differential Diagnosis: osteoblastoma, osteoid osteoma.

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Ameloblastic fibroma: 9330/0

It occurs in a young patient (l-2decades), slightly more in male, most commonly 70% in posterior mandible,
50%of cases are associated with the unerupted tooth, the lesion may be asymptomatic or have jaw swelling. Has some
recurrence tendency

Radiographic: appears as unilocular or multilocular radiolucency with well defined or

sclerotic margins. It may extend to ascending ramus associated with an impacted tooth.

Histopathology: The lesions contain thin strands that resemble enamel organ and cords
of odontogenic epithelium without microcyst formation. The mesenchymal tissue
resembles dental papilla (stellate and ovoid cells in a loose matrix), no collagen.

Treatment: more than local excision, associated recurrence70%. About 50%

may become amelo.fibrosarcoma.

Odontoma: 9280/0
The most common type of odontogenic tumors. It is a hamartomatous (noncancerous tumor made of an
abnormal mixture of normal tissues and cells from the area in which it grows ) and not true neoplasm. It divided into
two types; complex and compound odontoma.
Most cases are asymptomatic and diagnosed after a routine x-ray, or as a reason of the failure of a tooth to
erupted at the young patient (mean age 14y), they may occur at any sited tooth bearing area. However complex type
reported more common in the molar region and compound type at maxillary anterior.

Radiograph:
The compound type appears as a collection of a tooth-like structure of various size and shape surround by radiolucent
zone.
Complex odontoma is a calcified mass with radio-density of tooth structure also surround by narrow radiolucent rim
associated with an unerupted tooth.
Differential diagnosis: osteoma and other calcified bone lesion.

Histopathology:
Compound type it consists of multiple structures resembling small, single-rooted teeth contained in a loose fibrous
matrix and the enamel, dentin, and pulpal tissue are arranged in an orderly pattern.
Complex type composed of a single, disorganized mass of enamel, dentin, and pulp with no recognizable tooth shapes,
a thin layer of cementum often presents at the periphery of the mass.
Occasionally dentigerous cyst may arise from the epithelial lining of the fibrous capsule of complex odontoma.

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Compound type Complex type

Treatment: local excision, excellent prognosis.

Clear cell odontogenic tumor (carcinoma): 9341/3

It is a malignant neoplasm. The clear cytoplasm referred to glycogen-rich pre-secretary ameloblast cells.
Clinically patient is more than 50yrs, at maxilla or mandible; appear as painful swelling or some time symptom-free.

Radiograph: unilocular or multilocular radiolucency with irregular margins.

Histopathology: It is poorly circumscribed and consists mainly of clear cells with a central nucleus and a well-
defined cell membrane. They form large sheets, separated by thin fibrous septa, may be seen areas of hemorrhage.
Nervous tissue may be enveloped, and the tumor invades surrounding tissues.

Differential diagnosis: Intraosseous mucoepidermoid carcinoma with clear cells, calcifying epithelial odontogenic
tumor with clear cells, Metastatic clear cells neoplasm of renal carcinoma.
Treatment: Surgical radical resection+ radiotherapy, metastasis to lung and lymph
node. It has a high tendency to recur.

Ameloblastic carcinoma
It is a rare primary epithelial odontogenic malignant neoplasm. It is the malignant
counterpart of ameloblastoma.
Most cases arise in the posterior mandible, in patients aged > 45 years
It is aggressive lesions
Radiograph: longstanding lesions show poorly defined or irregularly marginated radiolucency, often with cortical
expansion, perforation, and infiltration into adjacent structures.
Histopathology: It shows the microscopic pattern of ameloblastoma with
cytologic features of malignancy (increased nuclear-to-cytoplasmic ratio,
nuclear hyperchromatism, and the presence of mitoses). Vascular or
perineural invasion, necrosis may all be present.
Prognosis:
1. aggressive clinical course

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2. perforation of the cortical plates of the jaw and

3. extension of the tumor into adjacent soft tissues, but metastases do not necessarily occur.

Biologic Classification of Odontogenic Tumors

BENIGN, NO RECURRENCE POTENTIAL BENIGN, SOME RECURRENCE POTENTIAL

Adenomatoid odontogenic tumor Cystic ameloblastoma

Squamous odontogenic tumor Calcifying epithelial odontogenic tumor
Cementoblastoma Central odontogenic fibroma
Periapical cementoosseous dysplasia Florid cementoosseous dysplasia
Odontoma Ameloblastic fibroma

BENIGN AGGRESSIVE MALIGNANT

Ameloblastoma Odontogenic carcinoma
1- Ameloblastic carcinoma
Clear cell odontogenic tumor 2- Primary intraosseous carcinoma
Odontogenic ghost cell tumor 3- Ghost cell odontogenic carcinoma
Odontogenic myxoma Clear cell odontogenic carcinoma
Odontogenic carcinosarcoma
Odontogenic sarcoma

Examples of questions
Odontogenic tumors that may have opaque foci (differential diagnosis):
Calcifying epithelial odontogenic tumor, Adenomatoid odontogenic tumor, Dentinogenic ghost cell tumor,
Odontoma, Cementoblastoma (immature).
Odontogenic tumors with multilocular radiolucencies (differential diagnosis):
Ameloblastoma, Ameloblastic fibroma, Odontogenic myxoma, Calcifying epithelial odontogenic tumor,
Central odontogenic fibroma.
Odontogenic tumors predominantly occurring in young patients:
Odontoma, Cementoblastoma, Ameloblastic fibroma, Adenomatoid odontogenic tumor.
What are the differences between complex & compound odontomas?
what is the origin of odontogenic tumors? And why these tumors appear only in the jawbones?
Why odontogenic myxoma has a high recurrence rate?
What are the biological subtypes of ameloblastoma?
What are the histopathological patterns of unicystic and polycystic ameloblastoma?
Why ameloblastic fibro-odontoma/fibrodentinoma terminology is not more used?
Name the disease/s with: Liesegang rings, driven-snow appearance, honeycomb pattern, soap-bubble
appearance.

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Notes:
1. Cemento-ossifying fibroma and cemento-osseous dysplasias are of odontogenic origin and are found in the
tooth bearing areas of the jaws
2. New benign epithelial odontogenic tumors entities were introduced;
1. sclerosing odontogenic carcinoma
2. primordial odontogenic tumor
3. adenoid ameloblastoma.

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